What is Shy Drager Syndrome?

Shy Drager Syndrome, which is known as multiple system atrophy, is a terminal phase of a neurological disorder which occurs rarely. It is a progressive disorder that affects the Central Autonomic Nervous System which kicks off during adulthood and worsens slowly. It also affects the person’s basal ganglia and brainstem. Basically it is the result of the autonomic nervous system’s failure to control the function of the body. It damages the person’s nerves which controls the involuntary function like heartbeat, blood pressure, breathing, and digestion. It is quite similar to the Parkinson’s disease since the persons with this syndrome manifest Parkinsonism like symptoms, which may be described further in this article. The main difference from Parkinson’s disease from this syndrome is that the damage in the person’s body is extensive when a person is diagnosed with this kind of syndrome.

Symptoms and Characteristics

Persons who have this syndrome may experience the following symptoms, signs and characteristics:

Weakness of the muscles

Ache in the neck

Constipation

Diarrhea

Loss of libido

Urinary incontinence or Inable to control bladder or bowel

Dizziness

Fatigue

Dry skin

Impotence

Impairment of the temperature regulatory system

Visual problems

Slurred speech

Poor balance

Stiffness

Movement is slow

Facial expression is lost

Dementia

Bradykinesia

Akinesia

Rigidity

Trouble swallowing

Reduce ability to sweat

Gasping

Sleep apnea periods

Abnormal posture

Confusion

Respiratory failure

Lightheadedness

Tremors (pill rolling tremors)

Difficulty in walking

Dementia

Depression

Shuffling

Myalgia

Mask appearance on the persons face

Unable to close the mouth

Frequent falling

Mild mental functioning decline

Nausea

Monotone

Slow speaking

Voice is on low volume

Orthostatic hypotension

Causes and Risk Factors

The exact causative agent or cause why this syndrome occurs is still unknown. However, recent studies show that it occurs on adulthood stage at around 50 years old and above. Also theories suggest that it may be related to the protein over production which is found in the person’s brain. While other theories suggest that it is primarily due to the degeneration of the nerve cell groups which is found in the person’s spinal column. Further more studies are conducted in determining the reason behind the said occurrences. That at risk for encountering or experiencing this syndrome falls to the ages in the middle to older persons. Aside from the age, the male gender is the one of greater risk to experiencing this syndrome. Rest assured that this syndrome is not of genetic cause and thus it can’t be inherited from one generation to the next.

Shy drager syndrome diagnosis:

In diagnosing the persons with Shy Drager Syndrome, physicians, who are either in the neurology or internal medicine experts, would require examinations such as:

Medical history examination

Physical examination

Eye examination

Neuromuscular examination

Vital signs such as pulse and blood pressure is an important assessment

Electroencephalogram examination

MRI examination of the person’s head

Barium Swallow examination

Sleep studies examination

CT scan examination

Plasma norepinephrine level examination

Blood examination

Urine examination

Treatment

There is no known cure for this syndrome and the treatments which are prescribing to persons who are diagnosed with this syndrome are medications which will only control the symptoms that the person manifests. These kinds of medications that may be prescribed are as follows:

Beta blockers such as propranolol which treats low blood pressure

Fludrocortisones which treats low blood pressure

MAOI which treats low blood pressure

Vasoconstrictors which treats low blood pressure

Vasopressin which treats low blood pressure

Medications to treat spasms of the bladder such as oxybutynin

Sildenafil which is a treatment for dysfunction of the male’s erectile

Corticosteroids such as prednisone

Immuno globulins which enhances the immune system

Aside from the medication, which is a form of dependent therapy, the persons with this syndrome are encouraged to follow independent therapy such as:

Eating small and frequent meals and those which are easier to swallow

Drinking caffeinated beverages every after meals

Avoiding alcoholic beverages

Moderate exercises

Increasing fluid and salt to increase blood pressure

Getting up slowly to avoid orthostatic hypotension

Wearing elastic abdominal binders and support stockings

Encouraging diet which are high in fiber and intake of fiber supplements

Aside from the ones that were mention, it is also advisable for the patient to undergo therapies such as:

Speech therapies

Tube feeding therapies for swallowing problems

Intermittent catheterization therapy for problems with the urination

Joining a support group therapy

Implantation of the penile for treatment of impotence

Breathing support therapy such as mechanical ventilation or oxygen therapy

Prognosis & Life Expectancy

The persons who are diagnosed with Shy Drager Syndrome have a poor outcome, hence a poor prognosis. Their physical functions as well as mental capabilities is loss in the long run and will eventually get even worse than before the disease started. There is a possibility of early death. The life expectancy with persons having Shy Drager Syndrome falls from the time and day she was diagnosed is from 7 up to 9 years.

Complications

The complications that are associated with these syndromes are as follows:

Injuries that are result from fainting or falling

Side effects of the medications such as hallucination or dizziness and the like

Difficulty in performance of the ADLs or activity of daily living

The loss of the ability to care or work which is seen progressively

Prevention

Since the cause of why Shy Drager Syndrome exists is still unknown, there are no known ways to prevent the occurrences of this kind of syndrome.